Treatment algorithm

Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups: see disclaimer

ACUTE

acute airway obstruction (without tissue diagnosis)

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secure airway + radiotherapy + corticosteroids

Airway should be secured by intubation or surgically first.

Urgent treatment with radiotherapy and corticosteroids should be used only for life-threatening situations. It should be deferred otherwise, due to interference with subsequent histopathological diagnosis.

Dose of corticosteroids should be limited and the dose decreased after 1 to 2 days of treatment or following symptomatic improvement.

Primary options

dexamethasone: 10 mg intravenous bolus initially, followed by 4 mg every 6 hours

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secure airway + percutaneous endovascular stenting

Airway should be secured by intubation or surgically first.

Becoming increasingly used, because the stent can be placed before a tissue diagnosis is available. It is a useful procedure for patients with severe symptoms such as respiratory distress that require urgent intervention.[21][22]​​ Meta-analyses have demonstrated that endovascular therapy with stenting has high technical and clinical success rates.[23][24][25]

Done percutaneously by obtaining access usually through the femoral vein. Performed under conscious sedation. Fluoroscopic guidance and iodinated contrast are used. Most operators use heparin during the procedure. [Figure caption and citation for the preceding image starts]: Post-dilatation of the superior vena cava stentImage obtained from cardiac catheterisation laboratory at University of Missouri, Columbia; used with permission [Citation ends].com.bmj.content.model.Caption@38625a07[Figure caption and citation for the preceding image starts]: Venography showing superior vena cava stenosis. Stent placement in the left pulmonary artery is seenImage obtained from cardiac catheterisation laboratory at University of Missouri, Columbia; used with permission [Citation ends].com.bmj.content.model.Caption@38c1e9f6[Figure caption and citation for the preceding image starts]: Percutaneous balloon angioplasty of the stenotic lesion in superior vena cavaImage obtained from cardiac catheterisation laboratory at University of Missouri, Columbia; used with permission [Citation ends].com.bmj.content.model.Caption@6c96fe38[Figure caption and citation for the preceding image starts]: Stent deployment in the superior vena cavaImage obtained from cardiac catheterisation laboratory at University of Missouri, Columbia; used with permission [Citation ends].com.bmj.content.model.Caption@1a16565e

Self-expanding or balloon-expandable stents may be used (usually bare metal stents).

Complications of percutaneous stenting are in the range of 3% to 7% and include volume overload due to sudden increase in preload, stent thrombosis, pulmonary embolus, stent migration, haematoma at the insertion site, infection, bleeding, and, very rarely, perforation or death.[22]

Patency rate is around 80% to 94%, and 20% of patients may require repeat stenting.

Bleeding risk is 1% to 14%, due to anticoagulation with aspirin, clopidogrel, and/or warfarin, which may be used following stent placement to prevent thrombosis.[21][31]

Data regarding post-procedural anticoagulation are lacking, and practices vary.[2]

ONGOING

malignant aetiology

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treatment of malignancy

Most malignant tumours causing superior vena cava (SVC) syndrome are sensitive to radiotherapy.

Chemotherapy is an effective option for treatment of lung cancer, lymphomas, and germ cell tumours.[26]

Thymomas that are resistant to chemotherapy and radiation may require surgical resection and SVC reconstruction (operative mortality rate of 5% and patency rate of 80% to 90%).[27]

Selection of therapy will depend on the type of malignancy, staging, and histopathology. See Small cell lung cancer, Non-small cell lung cancer, Non-Hodgkin's lymphomaThymic tumour.

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percutaneous endovascular stenting

Treatment recommended for ALL patients in selected patient group

Endovascular stenting is performed to achieve more rapid improvement in symptoms and has fewer side effects compared with radiotherapy.[23][24][25]​​

Undertaken percutaneously by obtaining access (usually) through the femoral vein. Performed under conscious sedation. Fluoroscopic guidance and iodinated contrast are used and most operators use heparin during the procedure.[Figure caption and citation for the preceding image starts]: Post-dilatation of the superior vena cava stentImage obtained from cardiac catheterisation laboratory at University of Missouri, Columbia; used with permission [Citation ends].com.bmj.content.model.Caption@3b82a2ee[Figure caption and citation for the preceding image starts]: Venography showing superior vena cava stenosis. Stent placement in the left pulmonary artery is seenImage obtained from cardiac catheterisation laboratory at University of Missouri, Columbia; used with permission [Citation ends].com.bmj.content.model.Caption@61980948[Figure caption and citation for the preceding image starts]: Percutaneous balloon angioplasty of the stenotic lesion in superior vena cavaImage obtained from cardiac catheterisation laboratory at University of Missouri, Columbia; used with permission [Citation ends].com.bmj.content.model.Caption@c63c32a[Figure caption and citation for the preceding image starts]: Stent deployment in the superior vena cavaImage obtained from cardiac catheterisation laboratory at University of Missouri, Columbia; used with permission [Citation ends].com.bmj.content.model.Caption@539c4a91

Self-expanding or balloon-expandable stents may be used (usually bare metal stents).

Complications of percutaneous stenting are in the range of 3% to 7% and include volume overload due to sudden increase in preload, stent thrombosis, pulmonary embolus, stent migration, haematoma at the insertion site, infection, bleeding, and, very rarely, perforation or death.[22]

Patency rate is around 80% to 94%, and 20% of patients may require repeat stenting.

Bleeding risk is 1% to 14%, due to anticoagulation with aspirin, clopidogrel, and/or warfarin, which may be used following stent placement to prevent thrombosis.[21][31] 

Data regarding post-procedural anticoagulation are lacking, and practices vary.[2]

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palliative therapy

Includes palliative radiotherapy, chemotherapy or corticosteroids (for lymphomas and thymomas), endovascular stents, or rarely bypass surgery, as it is invasive and difficult.[1]

In rare cases, surgical decompression can be performed.

Thrombolysis with indwelling catheters has also been described in small studies.[28]

Supportive treatment consists of diuretics, low-salt diet, avoidance of upper-extremity lines, head elevation, and oxygen.

Type of diuretic will depend on several factors including renal function and current or past history of diuretic use.

infectious aetiology

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treatment of infection

Underlying infection (e.g., aspergillosis, blastomycosis, histoplasmosis, nocardiosis) should be treated according to local sensitivities.

Choice of antimicrobial will depend on the underlying pathogen.

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palliative therapy

Endovascular stents and more rarely bypass surgery may be required if superior vena cava obstruction persists after treatment of infection.

iatrogenic aetiology

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catheter removal + thrombolysis and/or anticoagulation

Catheter(s) should be removed and local thrombolysis and/or short-course anticoagulation should be considered in these patients.[29]

Chronic warfarin therapy up to 1 year has been described in some reports.[32] Dose should be titrated to target INR of 2.0 to 3.0.

Thrombolysis has higher success rates if used within first 5 days of development of superior vena cava obstruction.[28]

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percutaneous balloon dilatation/stenting with or without lead removal

Percutaneous balloon dilatation/stenting is preferred in these patients.

Lead explantation may carry a high risk of mortality (1% to 3%).[30] Bypass surgery may be an option.

Infection of the leads should always be considered as a possibility and evaluated with blood cultures and transoesophageal echocardiogram.

Long-term anticoagulation with warfarin should be considered. Dose should be titrated to target INR of 2.0 to 3.0.

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Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups. See disclaimer

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